用达拉姆单抗成功治疗一名青少年的增生性肾小球肾炎伴单克隆 IgG 沉积。

IF 2.6 3区医学 Q1 PEDIATRICS Pediatric Nephrology Pub Date : 2024-12-01 Epub Date: 2024-06-11 DOI:10.1007/s00467-024-06425-2

Eva Svabova, Jakub Zieg, Martina Sukova, Eva Flachsova, Martin Kment, Vladimir Tesar

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引用次数: 0

摘要

增生性肾小球肾炎伴单克隆 IgG 沉积（PGNMID）是一种在儿童中非常罕见的疾病，目前尚无特效疗法。我们报告了一例 15 岁男孩的病例，他出现轻度肾功能减退和肾病综合征。肾活检显示，PGNMID伴有IgG3单克隆沉积和kappa轻链限制。流式细胞术显示外周血中有大量 CD38 浆细胞，但没有其他血液恶性肿瘤的迹象。患者接受了为期 6 个月的 Daratumumab（一种针对 CD38 的单克隆抗体）治疗。蛋白尿明显减少，肾功能恢复正常。基于在成人患者身上取得的积极经验，达拉土单抗也应在PGNMID儿童患者中进行研究。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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Proliferative glomerulonephritis with monoclonal IgG deposits in an adolescent successfully treated with daratumumab.

There is no specific treatment for proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID), a disease that is very rare in the pediatric population. We report the case of a 15-year-old boy who presented with mildly reduced kidney function and nephrotic syndrome. Kidney biopsy revealed PGNMID with monoclonal deposits of IgG3 with kappa light chain restriction. Flow cytometry showed a significant CD38 plasma cell population in the peripheral blood in the absence of other signs of hematological malignancy. The patient was treated with a 6-month course of daratumumab, a monoclonal antibody targeting CD38. There was a significant reduction in proteinuria and normalization of kidney function. Based on positive experience with adults, daratumumab should also be studied in children with PGNMID.

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来源期刊

Pediatric Nephrology 医学-泌尿学与肾脏学

CiteScore

4.70

自引率

20.00%

发文量

465

审稿时长

1 months

期刊介绍： International Pediatric Nephrology Association Pediatric Nephrology publishes original clinical research related to acute and chronic diseases that affect renal function, blood pressure, and fluid and electrolyte disorders in children. Studies may involve medical, surgical, nutritional, physiologic, biochemical, genetic, pathologic or immunologic aspects of disease, imaging techniques or consequences of acute or chronic kidney disease. There are 12 issues per year that contain Editorial Commentaries, Reviews, Educational Reviews, Original Articles, Brief Reports, Rapid Communications, Clinical Quizzes, and Letters to the Editors.